Mucous membrane pemphigoid-otorhinolaryngological manifestations: a retrospective cohort study

Ory Madgar, Avital Baniel, Noam Yarom, Eran Glikson, Tal Zeeli, Eli Sprecher, Eran E Alon
European Archives of Oto-rhino-laryngology 2020 January 24

INTRODUCTION: Mucous membrane pemphigoid (MMP) refers to a heterogeneous group of rare, chronic, inflammatory, mucous membrane-dominated, sub-epithelial blistering diseases that manifest with a varying constellation of oral, ocular, cutaneous, genital, nasopharyngeal, esophageal, and laryngeal lesions. MMP can progress to scarring in affected areas, which may lead to devastating complications including ocular involvement leading to blindness as well as laryngeal involvement leading to airway obstruction.

MATERIALS AND METHODS: A retrospective chart review was conducted for patients that were followed in two tertiary academic centers between 2009 through 2017 for upper aerodigestive tract manifestations of MMP. Patients with significant underlying skin involvement suggestive of bullous pemphigoid as well as MMP with isolated ocular involvement were excluded.

RESULTS: Twenty-seven patients were diagnosed with MMP and followed in two tertiary referral medical centers. The most common site of initial presentation was the oral cavity, and all patients had oral cavity involvement at some point. Two-thirds of our patients had complete remission or remission with intermittent relapses disease. Patients with mild to moderate disease showed excellent response to topical steroid treatment. For more severe disease, high-dose prednisone was insufficient, as in most cases relapse occurred at some point of time during tapering down.

CONCLUSIONS: MMP is a rare autoimmune disorder that may present with a wide spectrum of head and neck manifestations and, potentially, catastrophic sequelae. This work highlights the experience of two tertiary centers with MMP in an attempt to draw attention to this unusual disorder.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"